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Rheumatology Advance Access originally published online on February 10, 2009
Rheumatology 2009 48(4):363-366; doi:10.1093/rheumatology/ken505
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Male microchimerism and HLA compatibility in French women with sclerodema: a different profile in limited and diffuse subset

Justyna M. Rak1,*, Philippe P. Pagni1,*, Kiet Tiev2, Yannick Allanore3, Dominique Farge4,5, Jean-Robert Harlé6, David Launay7, Eric Hachulla7, Rémi Didelot8, Jean Cabane2, André Kahan3, Marielle Martin1, Brigitte Granel9, Jean Roudier1,10 and Nathalie C. Lambert1

1INSERM U639, Marseille, 2Service de Médecine Interne, Hôpital St Antoine, 3Service de Rhumatologie, Hôpital Cochin, 4Service de Médecine Interne et Pathologie Vasculaire, 5INSERM U697, Hôpital St Louis, Paris, 6Service de Médecine Interne, Hopital La Conception, Marseille, 7Service de Médecine Interne, Centre National de Référence des Atteintes Vasculaire de la Sclérodermie Systémique, Hôpital Claude Huriez, Lille, 8Centre d'Examen de Santé Assurance Maladie, 9Service de Médecine Interne, Hôpital Nord and 10Service de Rhumatologie, Hôpital La Conception, Marseille, France.

Correspondence to: Nathalie C. Lambert, Laboratoire Immunogénétique de la Polyarthrite Rhumatoïde, INSERM U639, Parc scientifique de Luminy, 163 avenue de Luminy, Bât TPR2 entrée A, 1er étage, 13288 Marseille, France. E-mail: nathalie.lambert{at}medecine.univ-mrs.fr


   Abstract

Objectives. Male microchimerism (Mc) persisting from pregnancy has been found at greater frequencies and/or higher quantities in women with scleroderma (SSc) compared with controls, suggesting a possible role in disease development. Moreover, women with an HLA-compatible child have a higher risk to develop SSc. We tested the hypothesis, on our French SSc cohort, that women with lcSSc and dcSSc, two distinct clinical subsets, have a different profile in terms of Mc and HLA compatibility in families.

Methods. We studied 98 women (52 lcSSc and 46 dcSSc) for male Mc, by real-time PCR, in their whole blood and/or peripheral blood mononuclear cells (PBMC). Similarly, 91 matched healthy women were analysed. Complete HLA-DRB1 typing was obtained for 58 SSc and 68 control families (proband/children).

Results. Women with lcSSc (N = 50) had male Mc more often in their whole blood than women with dcSSc (N = 40, 20 vs 5%, P = 0.038), but not significantly more than controls. By contrast, women with dcSSc (N = 36) hold Mc more often in PBMC (25 vs 9%), but not significantly and have greater quantities than controls (N = 82, P = 0.048). This contrast is also visible in feto-maternal HLA-DRB1 compatibility, which was increased only among women with lcSSc (N = 33) compared with controls (N = 68, P = 0.003).

Conclusion. For the first time, we showed that women with lcSSc and dcSSc hold male Mc in different blood compartments. Furthermore, a distinct pattern between the two SSc subtypes is observed for feto-maternal HLA-DRB1 compatibility. These results suggest a different mechanism behind each type of disease.

KEY WORDS: Microchimerism, Limited cutaneous scleroderma, Diffuse cutaneous scleroderma, Blood, Peripheral blood mononuclear cells, Y-chromosome


*Justyna M. Rak and Philippe P. Pagni equally contributed to this work.

Submitted 30 June 2008; revised version accepted 15 December 2008.
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